Definition and general features:
• It is an autoimmune disease characterized by progressive damage of medium and small intrahepatic bile ducts.
• Primary biliary cirrhosis/cholangitis (PBC) mainly affects women (90%) and usually presents clinically in middle age.
Pathology:
• There is lymphocytic infiltration and granulomatous damage (fluoride duct lesions) in medium and small-sized intrahepatic bile ducts, resulting in periportal fibrosis.
• Fibrosis progresses to cirrhosis.
Clinic:
• There is cholestasis due to impaired bile flow.
• Fatigue is the most common symptom in the early period.
• Itching is the most important symptom suggestive of hepatobiliary disease.
• Fat malabsorption and weight loss may occur.
• Some patients have xanthoma and xanthelasma.
• It is most often accompanied by Sjögren's syndrome (keratoconjunctivitis sicca). Many other autoimmune diseases and findings can also be seen together.
Diagnosis:
• Cholestasis enzymes and direct bilirubin increased.
• Bilirubin level is the most important determinant of prognosis.
• Serum IgM levels are increased.
• Antimitochondrial antibodies (AMA} are positive in the vast majority of patients.
• Cholesterol levels have increased.
• Bile ducts are expected to be normal in USG. It is important in differentiating it from pathologies that cause extrahepatic cholestasis.
• MRCP and ERCP may be useful in differentiating from extrahepatic cholestatic diseases.
In PBK, no pathology is expected in the bile ducts in MRCP and ERCP.
• Definitive diagnosis is made by demonstrating intrahepatic bile duct damage in liver biopsy.
Treatment:
• Ursodeoxycholic acid is the first choice in treatment. It slows the course of the disease and can reduce the symptoms. Obeticholic acid is recommended in patients who do not respond to ursodeoxycholic acid therapy.
• Cholestyramine: It is used in the treatment of itching.
• Immunosuppressive treatments have no place.